[Hereditary and non-hereditary cutaneous amyloidoses]

Pathologe. 2009 May;30(3):197-204. doi: 10.1007/s00292-009-1130-7.
[Article in German]

Abstract

Amyloid and amyloidosis describes a heterogeneous group of diseases which are characterized by the pathological extracellular deposition of autologous proteins. Basically, amyloidoses can be divided into systemic or organ-limited (e.g. cutaneous) forms and can be acquired or hereditary in nature. The subclassification discriminates between primary amyloidosis (in the absence of an obvious predisposing disease) and secondary amyloidosis (if caused by a certain underlying disease). The subclassification of amyloidoses is based on the main protein constituent and therefore on the chemical composition of the amyloid fibrils. However, the exact etiopathogenesis of amyloid formation remains unclear. In addition to the clinical presentation, histology, electron microscopy and biochemical-immunological differentiation are also decisive for a proper diagnosis. In cutaneous amyloidosis the deposition of amyloid either occurs along reticulin fibers and the basal membrane (perireticulary amyloidoses) or along collagen fibers (pericollagenous amyloidosis). The purpose of this article is to provide an up-to-date overview on the different kinds of cutaneous amyloidoses.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Amyloid / analysis
  • Amyloid / ultrastructure
  • Amyloidosis / classification
  • Amyloidosis / genetics
  • Amyloidosis / pathology*
  • Amyloidosis, Familial / classification
  • Amyloidosis, Familial / genetics
  • Amyloidosis, Familial / pathology*
  • Basement Membrane / pathology
  • Diagnosis, Differential
  • Humans
  • Microscopy, Electron
  • Skin / pathology
  • Skin Diseases, Genetic / classification
  • Skin Diseases, Genetic / genetics
  • Skin Diseases, Genetic / pathology*

Substances

  • Amyloid